14 results
ANTI-MDA5 DERMATOMYOSITIS

Cutaneous manifestations:
• Periorbital heliotrope (blue-purple) rash with edema
• Erythematous rash on the face, or the
(blue-purple) rash ... • Erythematous rash ... dorsal part of the MCP ... severity of the disease ... #rheumatology #
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation ... Petechial or purpuric rash ... with leukopenia, anemia ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
, dermatomyositis ... vein thrombosis Hematologic ... lymphopenia, low PLT • Anemia ... La, anti-Jo-1, SCL ... Chronic B-cell activation
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Lymphohistiocytosis (HLH) Pathophysiology ... →Activation of CD8 ... IFN-y) → Excessive activation ... • Autoimmune diseases ... Lymphohistiocytosis #diagnosis #management
Oroya Fever
Hematologic disease caused by Bartonella bacilliformis
Restricted to the Andes highlands of Peru & Ecuador
B. bacilliformis:
Oroya Fever Hematologic ... disease caused ... severe hemolytic anemia ... coinfection, toxo reactivation ... Fever #diagnosis #management
Adult Onset Still's Disease
Systemic inflammation with urticarial rash.
Rash: 
 • Nonpruritic, evanescent (correlates with time of
Onset Still's Disease ... with urticarial rash ... Ferritin >3000 ng/mL ... AOSD #diagnosis #rheumatology ... #management
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
excessive macrophage activation ... age, however the disease ... Ferritin >500 ng/mL ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
(blue-purple) rash ... • Erythematous rash ... dorsal part of the MCP ... had more severe disease ... #diagnosis #management
Stiff Person Syndrome (SPS) - Diagnosis and Management Summary

Epidemiology:
 • Prevalence: 1-2 cases per million -
Diagnosis and Management ... and pernicious anemia ... Person Syndrome Pathophysiology ... Anti-GAD Ab (>1000u/ml ... SPS #Diagnosis #Management
Hodgkin's Lymphoma Overview

Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
Weight loss • Anemia ... Immune hemolytic anemia ... number of sites of disease ... classification #hematology ... #oncology #management